Paediatric Haematology and Oncology, University of California, San Francisco, USA; Paediatric Allergy, Immunology, and Bone Marrow Transplantation, University of California, San Francisco, USA.
Paediatric Haematology and Oncology, University Hospital Muenster, Germany; NHL-BFM Study Center, University Hospital Muenster, Germany.
Best Pract Res Clin Haematol. 2023 Mar;36(1):101449. doi: 10.1016/j.beha.2023.101449. Epub 2023 Feb 16.
Lymphoblastic lymphoma (LBL) is the second most common type of non-Hodgkin Lymphoma (NHL) in children, adolescents, and young adults (CAYA), accounting for 25-35% of all cases. T-lymphoblastic lymphoma (T-LBL) comprises 70-80% of cases, while precursor B-lymphoblastic lymphoma (pB-LBL) makes up the remaining 20-25% of cases. Event-free and overall survival (EFS and OS) for paediatric LBL patients both exceed 80% with current therapies. Treatment regimens, especially in T-LBL with large mediastinal tumours, are complex with significant toxicity and long-term complications. Though prognosis overall is good for T-LBL and pB-LBL with upfront therapy, outcomes for patients with relapsed or refractory (r/r) disease remain dismal. Here, we review new understanding about the pathogenesis and biology of LBL, recent clinical results and future directions for therapy, and remaining obstacles to improve outcomes while reducing toxicity.
淋巴母细胞淋巴瘤（LBL）是儿童、青少年和年轻成人（CAYA）中第二大常见的非霍奇金淋巴瘤（NHL），占所有病例的 25-35%。T 淋巴母细胞淋巴瘤（T-LBL）占病例的 70-80%，而前体 B 淋巴母细胞淋巴瘤（pB-LBL）占剩余的 20-25%。目前的治疗方法使儿童 LBL 患者的无事件生存（EFS）和总生存（OS）均超过 80%。治疗方案，特别是伴有大纵隔肿瘤的 T-LBL，具有复杂性、显著毒性和长期并发症。尽管初始治疗后 T-LBL 和 pB-LBL 的总体预后良好，但复发或难治性（r/r）疾病患者的预后仍然很差。在这里，我们回顾了 LBL 的发病机制和生物学、最近的临床结果和治疗的未来方向，以及在降低毒性的同时提高疗效的剩余障碍。
